Aicardi syndrome

Case contributed by Dr Ammar Haouimi

Presentation

Refractory partial epilepsy. The ophthalmological exam revealed chorioretinal lacunae.

Patient Data

Age: 5 years
Gender: Female
MRI

Left retrocerebellar extra-axial cystic lesion with mass effect on the cerebellar parenchyma, and thinning of the adjacent inner table of the skull. Partial dysgenesis of the corpus callosum with dilated right lateral ventricle. Right peritrigonal heterotopia.

Moderate tectal enlargement. Cysts of the right choroid plexus and pineal region. Area of high signal intensity FLAIR, and T2WI of the left lentiform nucleus. The dysplastic cortex of the right parasagittal occipital region with the small interhemispheric cyst.

Case Discussion

The MRI features and the presence of chorioretinal lacunae on the ophthalmological exam are highly suggestive of Aicardi syndrome.

Additional contributor: R. Bouguelaa, MD.

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Case information

rID: 66029
Published: 16th Feb 2019
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included
Institution: Aurès Medical Imaging Center - Batna Algeria

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